title: Management of biliary atresia in the liver transplantation era: a 15-year, single-center experience.
abstract: OBJECTIVE
  The aim of this study was to address the perioperative aspects of hepatoportoenterostomy (HPE) for biliary atresia (BA), through the study of a 15-year, single-center experience of the management of this disease.
  
  
  METHODS
  One hundred twenty-seven patients were divided into three groups, depending on the variant of HPE performed: group A (n = 53) underwent HPE with external diversion of the Roux-en-Y anastomosis; group B (n = 54) underwent HPE with a long (35 to 40 cm) Roux-en-Y anastomosis, without diversion; and group C (n = 20) underwent the same kind of HPE as group B, but with a modified, "super extensive" dissection of the porta hepatitis. Eleven children in group B had an intussusception type antireflux valve in the Roux-en-Y loop. The porta hepatitis of 105 children was histologically classified in types I to III and correlated with rate of postoperative bile flow and age at surgery. Liver transplantation was performed after HPE in 20 patients.
  
  
  RESULTS
  Overall, biliary drainage was achieved in 72.5% of the children after HPE and 26.8% of all patients became jaundice free. Porta hepatitis type III was associated with a significantly higher rate of biliary drainage post-HPE then types I and II. There was no difference in the rate of bile drainage, relative number of jaundice-free patients, and mean number of episodes of cholangitis per year among surgical groups A, B, C. In group A, 43.7% of the patients had complications related to the stoma. The actuarial survival of children who underwent HPE followed by liver transplantation was 85%.
  
  
  CONCLUSIONS
  (1) There is no correlation between type of porta hepatis and age at surgery for BA; (2) type III porta hepatis is associated with higher rates of bile drainage post-HPE; (3) children older than 16 weeks with BA should still be considered for HPE; (4) in these older patients, factors other than the type of porta hepatis, possibly the degree of liver damage, play a role in the lower rate of bile drainage observed; (5) external diversion of the Roux-en-Y in HPE is not beneficial and is detrimental because of stoma-related complications; (6) an antireflux valve in the Roux-en-Y loop does not reduce the incidence of cholangitis post-HPE; (7) Surgical reexploration does not restore biliary drainage; and (8) sequential therapy with HPE followed by liver transplantation remains the treatment of choice for BA.
authors.affiliation: Division of Surgery, Children's Institute, University of São Paulo Medical School, Brazil.
authors.forename: J G
authors.initials: JG
authors.lastname: Maksoud
authors.forename: D O
authors.initials: DO
authors.lastname: Fauza
authors.forename: M M
authors.initials: MM
authors.lastname: Silva
authors.forename: G
authors.initials: G
authors.lastname: Porta
authors.forename: I
authors.initials: I
authors.lastname: Miura
authors.forename: C N
authors.initials: CN
authors.lastname: Zerbini
mesh_terms: D000206:Actuarial Analysis; D000713:Anastomosis, Roux-en-Y / Q000379:methods; D001656:Biliary Atresia / Q000453:epidemiology / Q000601:surgery*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004322:Drainage; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D011171:Portoenterostomy, Hepatic / Q000379:methods; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome